Progressive systemic sclerosis (PSS), also termed scleroderma, is a disease characterized by fibrosis in skin, blood vessels and visceral organs, especially in the gastrointestinal system, heart and kidneys. Although most patients have long survival, some of them progress rapidly to death. Pulmonary system involvement and pulmonary hypertension are the most frequent causes of death. Preoperative assessment is very important in the management of anesthesia, as there is an increased risk of difficult intubation in association with involvement of joints and skin and aspiration risk. In this article, we aimed to present the anesthetic management in a case of a 67-year-old male with PSS disease who had an amputation surgery, together with a literature review.